Herridge M S, Pearson F G, Downey G P
Department of Medicine, Division of Respiratory Diseases, University of Toronto, Ontario, Canada.
J Thorac Cardiovasc Surg. 1996 May;111(5):961-6. doi: 10.1016/s0022-5223(96)70371-9.
Wegener's granulomatosis frequently involves the subglottis and trachea, often leading to compromise of the upper airway. Moreover, the stenotic segments may persist or progress despite control of the disease elsewhere in the body. In this report, we describe the cases of five patients with Wegener's granulomatosis who, in addition to nasal, sinus, pulmonary and renal involvement, had symptomatic subglottic or tracheal stenosis. Biopsy specimens from involved sites in the subglottis and trachea were often not diagnostic, and the diagnosis was later confirmed by a positive antineutrophil cytoplasm antibody titer. All patients had clinical remission on standard therapeutic regimens with prednisone and cyclophosphamide but continued to have symptoms of extrathoracic airway obstruction. Three of the five patients underwent primary thyrotracheal anastomosis while their disease was in clinical remission, without postoperative compromise of anastomotic integrity or wound healing despite concurrent use of prednisone and cyclophosphamide. There has been no evidence of local disease recurrence during follow-up periods ranging from 3 months to 14 years. We conclude that surgical intervention is a viable treatment option for patients who have symptomatic stenotic segments of the subglottis and trachea as a result of Wegener's granulomatosis in clinical remission.
韦格纳肉芽肿常累及声门下区和气管,常导致上呼吸道梗阻。此外,尽管身体其他部位的疾病得到控制,但狭窄段可能持续存在或进展。在本报告中,我们描述了5例韦格纳肉芽肿患者的病例,这些患者除了有鼻、鼻窦、肺和肾受累外,还出现了有症状的声门下或气管狭窄。来自声门下和气管受累部位的活检标本往往无法确诊,后来抗中性粒细胞胞浆抗体滴度呈阳性证实了诊断。所有患者在使用泼尼松和环磷酰胺的标准治疗方案后临床缓解,但仍有胸外气道梗阻症状。5例患者中有3例在疾病临床缓解期接受了一期甲状腺气管吻合术,尽管同时使用泼尼松和环磷酰胺,但术后吻合口完整性和伤口愈合均未受到影响。在3个月至14年的随访期内,没有局部疾病复发的证据。我们得出结论,对于因韦格纳肉芽肿处于临床缓解期而出现有症状的声门下和气管狭窄段的患者,手术干预是一种可行的治疗选择。